Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin

Electrophysiological characterization of adult-onset Niemann–Pick type C disease

(2015) Rosa Iodice et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease

(2015) Charles H. Vite et al.   Science Translational Medicine

The molecular, cellular, and morphological components of blood–brain barrier development during embryogenesis

(2015) Nellwyn Hagan et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Defects in the retina of Niemann-pick type C 1 mutant mice

(2014) Xin Yan et al.   BMC NEUROSCIENCE

Collaborative Development of 2-Hydroxypropyl-β-Cyclodextrin for the Treatment of Niemann-Pick Type C1 Disease

(2014) Elizabeth Ottinger et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Diagnostic value of visual evoked potentials for clinical diagnosis of multiple sclerosis

(2014) Niphon Chirapapaisan et al.   DOCUMENTA OPHTHALMOLOGICA

Preferred EEG brain states at stimulus onset in a fixed interstimulus interval equiprobable auditory Go/NoGo task: A definitive study

(2014) Robert J. Barry et al.   INTERNATIONAL JOURNAL OF PSYCHOPHYSIOLOGY

Hearing Loss is an Early Consequence of Npc1 Gene Deletion in the Mouse Model of Niemann–Pick Disease, Type C

(2014) Kelly A. King et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

A marked paucity of granule cells in the developing cerebellum of the Npc1−/− mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin

(2014) S. Nusca et al.   NEUROBIOLOGY OF DISEASE

The role of nitric oxide on visual-evoked potentials in MPTP-induced Parkinsonism in mice

(2014) Sinem Aras et al.   NEUROCHEMISTRY INTERNATIONAL

Atypical multisensory integration in Niemann-Pick type C disease – towards potential biomarkers

(2014) Gizely N Andrade et al.   Orphanet Journal of Rare Diseases

Early maternal separation: a rodent model of depression and a prevailing human condition

(2014) Jerzy Vetulani   Pharmacological Reports

Olfactory Deficits in Niemann-Pick Type C1 (NPC1) Disease

(2014) Marina Hovakimyan et al.   PLoS One

A mouse model of higher visual cortical function

(2013) Lindsey L Glickfeld et al.   CURRENT OPINION IN NEUROBIOLOGY

Auditory Phenotype of Niemann-Pick Disease, Type C1

(2013) Kelly A. King et al.   EAR AND HEARING

Npc1 Acting in Neurons and Glia Is Essential for the Formation and Maintenance of CNS Myelin

(2013) Ting Yu et al.   PLoS Genetics

Comparison of Visual Evoked Potentials and Retinal Nerve Fiber Layer Thickness in Alzheimer’s Disease

(2013) Robert Kromer et al.   Frontiers in Neurology

Enhanced brain performance in mice following postnatal stress

(2012) Alberto Loizzo et al.   JOURNAL OF ENDOCRINOLOGY

Hearing Loss and Hair Cell Death in Mice Given the Cholesterol-Chelating Agent Hydroxypropyl-β-Cyclodextrin

(2012) Mark A. Crumling et al.   PLoS One

Glutamatergic neurotransmission in a mouse model of Niemann–Pick Type C Disease

(2011) Giovanna D'Arcangelo et al.   BRAIN RESEARCH

Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations

(2011) Charina M. Ramirez et al.   JOURNAL OF LIPID RESEARCH

Anatomically Defined Neuron-Based Rescue of Neurodegenerative Niemann-Pick Type C Disorder

(2011) M. E. Lopez et al.   JOURNAL OF NEUROSCIENCE

Unesterified Cholesterol Accumulation in Late Endosomes/Lysosomes Causes Neurodegeneration and Is Prevented by Driving Cholesterol Export from This Compartment

(2011) A. Aqul et al.   JOURNAL OF NEUROSCIENCE

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice

(2011) Sarah N.R. Pressey et al.   NEUROBIOLOGY OF DISEASE

Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding

(2011) M. S. Deffieu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stress- and Allostasis-Induced Brain Plasticity

(2010) Bruce S. McEwen et al.   Annual Review of Medicine

Niemann-Pick disease type C

(2010) Marie T Vanier   Orphanet Journal of Rare Diseases

2-Hydroxypropyl-β-Cyclodextrin Raises Hearing Threshold in Normal Cats and in Cats With Niemann-Pick Type C Disease

(2010) Sarah Ward et al.   PEDIATRIC RESEARCH

Weekly Cyclodextrin Administration Normalizes Cholesterol Metabolism in Nearly Every Organ of the Niemann-Pick Type C1 Mouse and Markedly Prolongs Life

(2010) Charina M Ramirez et al.   PEDIATRIC RESEARCH

Does the brain connect before the periphery can direct?

(2009) Brian K. Hoffpauir et al.   BRAIN RESEARCH

Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol

(2009) Hyock Joo Kwon et al.   CELL

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

(2009) Benny Liu et al.   JOURNAL OF LIPID RESEARCH

Lack of Niemann–Pick type C1 induces age-related degeneration in the mouse retina

(2009) Thomas Claudepierre et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

(2009) Cristin D. Davidson et al.   PLoS One

Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse

(2009) B. Liu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lysobisphosphatidic Acid Controls Endosomal Cholesterol Levels

(2008) Julien Chevallier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice

(2008) Hao Li et al.   JOURNAL OF LIPID RESEARCH

Brainstem neuropathology in a mouse model of Niemann–Pick disease type C

(2008) Zhuo Luan et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES